Childhood ocular tumor Childhood ocular tumor Childhood ocular tumor Childhood ocular tumor

1. CHILDHOOD OCULAR TUMOR
AND MANAGEMENT
Dr. Md. Almas Hossain
Associate Professor
Department of Ophthalmology
Sylhet MAG Osmani Medical College.

2. Childhood Ocular Tumors
► Intra or Extra ocular (Orbit & Lid) .
► May be benign or malignant .
► Benign tumors are much more common than malignant
tumors.
► Many tumors of childhood show variable qualities and
sometimes difficult to categories .
► The overall incidence of childhood ocular malignancy is
more during the first five years of life.

3. Common presentations of childhood
ocular tumors
• Swelling and discoloration of eye lids.
• Proptosis or other displacement of the globe.
• Ptosis.
• Strabismus (squint).
• Impairment of vision.

4. Primary malignant neoplasm
► Arises from orbital tissue elements e.g. – Sarcomas
(Rhabdomyosarcoma).
► Tumors of epithelial origin e.g. – Ca. of Lacrimal
glands.

5. RHABDOMYOSARCOMA
• Most common primary paediatric orbital malignancy.
• Average age of onset is 5 – 7 yrs.
• 5% before the age of 1 yr.
• 15% after 10 yrs.
• 90% occurs before the age of 16 yrs.
• Prognosis – good if treated properly.

6. Fig: Rhabdomyosarcoma (RE)

7. C/F of Rhabdomyosarcoma
 Proptosis – developed rapidly (within few days).
 Ptosis & squinting also common.
Differential Diagnosis:
● Orbital cellulites.
● Granulocytic sarcoma.
 CT & MRI – Irregular but well circumscribed mass of
uniform density.
 Biopsy is required for the confirmation.

8. CT scan of Rhabdomyosarcoma (LE)

9. Treatment of Rhabdomyosarcoma
 Small encapsulated or well localized Rhabdomyosarcoma
should be totally excised.
 For larger & more extensive cases – Chemo & radiotherapy.
► The prognosis depends on the stage & location of disease
at the time of diagnosis.
● Patients with tumors localized to the orbit have approximate
95% cure rate.

10. CHILDHOOD METASTATIC TUMORS
Neuroblastoma
► Most common childhood cancer.
► Frequent source of orbital metastasis.
► Originates in either adrenal gland or sympathetic ganglion chain in the
retroperitoneal mediastinum.
► Metastatic Neuroblastoma in the orbit typically produces proptosis, &
periorbital ecchymosis.
► CT – shows evidence of bone destruction.
► With intensive treatment including radio & chemotherapy prognosis is
considerably better in infants under the age of 1 yr. than older children.

11. Fig. Neuroblastoma RE

12. Wilms Tumor
► Though most common childhood cancer but rarely
metastasizes from the kidney to orbit.
Leukemia
► Most common malignancy of childhood.
► Leukemic cells causing proptosis, lid swelling &
ecchymosis. If infiltration of the optic nerve by leukemic
cells may cause papilloedema even loss of vision.

13. CHILDHOOD BENIGN ORBITAL TUMORS
Capillary Haemangioma:
► Most common benign orbital & periorbital tumors in
childhood.
► Female child more affected than male child (2:1).
► Skin has a characterized raised , red dimpled appearance
(strawberry nevus).
► 90% are recognized by the age of 6 month.

14. Cont-2 (Capillary Haemangioma)
► Maximum size usually raised between 6 to 12 months of age.
► Spontaneous involution begins during the second year of life.
► Regression is about 40% by the age of 4 yrs & 80% by age
of 8 yrs.
► Approximately half of the children with eyelid & orbital
haemangioma develop some degree of amblyopia resulting
form anisometropic astigmatism.

15. Fig. Capillary Haemangioma (RE)

16. LYMPHANGIOMA
► Most common orbital tumor of vascular origin is childhood.
► Lesion consists largely of lymph filled channels lined by endothelium.
► Complete excision is difficult.
 No treatment is indicated when the only symptom is mild
proptosis. Surgical evacuation should be considered when CT
or MRI shows large blood filled cavities indicating acute
hemorrhagic deterioration.

17. Ectopic Tissue Mass
► Epidermoid cyst .
► Limbal dermoid.
► Teratomas.
► Ectopic lacrimal gland.
► Encephalocele or meningocele.

18. Fig- Limbal Dermoid LE

19. TREATMENT OF CHILDHOOD BENIGN
ORBITAL TUMORS
Indication:
■ To prevent development of amblyopia.
■ To prevent optic nerve compression.
■ To prevent exposure keratopathy.
Treatment Method:
► Intralesional injection of corticosteroids 1-2ml of short &
long acting agents (Betamethasone 6mg/ml and
Triamcinolone 40mg/ml) combined 1:1 mixture.
► Oral Corticosteroids (Prednisolone 2mg/kg/day)
alternately may also be effective treatment.
• Shrinkage usually during the first to second week of
treatment start.

20. CHILDHOOD INTRAOCULAR
BENIGN TUMOTRS
Though benign intraocular tumor is less common but some of
tumors are found as bellows:
● Congenital hypertrophy of RPE.
● Combined hamartoma of the retina and RPE.
● Uveal nevus.
● Hemangioma of the choroid and retina.

21. CHILDHOOD INTRAOCULAR
MALIGNANT TUMOTRS
Retinoblastoma
Most common primary intraocular malignant tumor of
childhood originating in the outer nuclear layer of the retina &
one of the most common of all pediatric solid tumors (3%) with
an incidence of about 1:2000 live birth.

22. * Positive family history 6% & usually bilateral.
* Sporadic 94% (Unilateral).
* It is autosomal dominant.
* Usually diagnosed during the first yr of life.
* Onset later than 7 yrs of age is rare.
* Most common initial sign is leukocoria (white pupillary
reflex ) 60%, first noticed by the family.
* Squinting 20% cases, so fundus examination is mandatory in
squinting pt. of childhood.
Inheritance of Retinoblastoma

23. ► Visual impairment.
► Hyphaema.
► Vitreous Hge.
► Glaucoma.
► Orbital inflammation.
Common Presentation
of Retinoblastoma

24. Type of Retinoblastoma
Endophytic Retinoblastoma
- Associated with vitreous seeding.
- Grows inwards towards the vitreous cavity prosecuting from the
retinal surface as a white, cottage cheese like mass with surface
blood vessels.
Exophytic Retinoblastoma
- Associated with sub-retinal fluid accommodation.
- Grows outwards as a sub-retinal multi lobulated white mass,
causing RD.

25. STAGES OF RETINOBLASTOMA
► Quiescent stage (6-12 month).
► Glaucomatous stage.
► Stage of extra ocular extension.
► Stage of metastasis.

26. Spread of Retinoblastoma
Direct spread
► To intra ocular tissue.
► Into the extra ocular tissue.
● Into CNS
● Into orbit
Lymphatic spread
► Along the lymph channels pre ocular with cervical lymph nodes.
By blood stream
► Choroidal invasion is the main route.
► Common site are Bones & Liver.
► Lung is the least common site.

27. INVESTIGATION OF RETINOBLASTOMA
* Indirect ophthalmoscopy with scleral indentation & pupil must be
dilated with mydriatics and both eyes should be checked.
Special Investigations :
* Ultrasonography – B scan Asses tumor size and calcification within the
tumor.
* CT scan- Demonstrates gross involvement of the optic nerve, orbital &
CNS extension.
* Systemic Investigations:
. Bone marrow aspiration.
. Lumber puncture.
Only perform when patient with optic nerve involment or evidence of
extra ocular extension.

28. Fig. Retinoblastoma LE

29. CT Scan showing advanced Retinoblastoma BE

30. TREATMENT OF RETINOBLASTOMA
(Related to tumor size, location)
Associated findings –
* RD
* Sub retinal & Vitreous tumor seeds.
* The state of the fellow eye.
Small tumor - < 4mm diameter & 2mm thickness without
vitreous seeds or sub retinal seeds can be treated with trans-
pupillary thermo leaser or chemotherapy.

31. Medium size tumors-
* Bracytheraphy- indicated for the tumor size < 12 mm dia
and 6 mm thickness.
* Chemotherapy with carboplatin, vincristin & etoposide
may be combined with cyclosporine in IV route, 3 cycle
over 4 to 9 months period depending on disease severity.
Large tumors-
* Chemotherapy to shrink the tumor ( Chemo reduction).
* Enucleation with long piece of optic nerve (8-12mm)
- If chemo reduction fails.

32. If extra ocular extension-
- beyond the lemina cribosa is treated with chemotherapy
after enucleation.
- Extension to the cut end of the optic nerve or extension
through the sclerais treated with chemo & radiotherapy
of the affected orbit.
Incase of metastatic condition-
Treated with high dose chemotherapy. Patient with malignant
cells in the CSF may required intrathecal methotrexate.

33. Prognostic Factors of Retinoblastoma
The overall mortality rate is 2 to 5% & related to the followings:
* Tumor size & location.
* Cellular differentiation.
* Optic nerve involvement.
* Invasion.
* Extrascleral spread  Most grave prognosis.

34. DIFFERENTIAL DIAGNOSIS OF
RETINOBLASTOMA
* Persistent hyperplastic primary vitreous.
* Coats Disease.
* Retinopathy of prematurity.
* Toxocariasis.
* Retinal Dysplasia.

35. Thanks all